Recurrent Sacral Chordoma: A Case Report

Abstract

Chordoma is a rare and locally aggressive tumor that arises from the notochordal remnants and has an incidence of 0.1/100000 per year. It has a predilection for the axial skeleton and is the most common primary malignant tumor of sacrum. The mainstay of treatment is wide surgical excision but there is a risk of recurrence due to the infiltrating nature of the tumor. Here, we report a case of a 56-years male who complained of pain over his sacral region for the past two years along with episodic urinary symptoms, constipation, and weakness of both legs. Seven years after undergoing surgery and radiotherapy for his sacral chordoma, he was diagnosed with recurrent sacral chordoma and planned for reoperation. Subtotal excision of the chordoma was done which significantly alleviated his symptoms postoperatively. Timely intervention helps to improve the quality of life in patients with either primary or recurrent sacral chordomas.

Partial Sacrectomy in the Management of Sacral Chordoma: Case Report and Literature Review

“…1 Therefore, these tumors are often diagnosed incidentally or at an advanced stage, with destruction of adjacent structures and soft tissue invasion. 4 The main differential diagnosis is chondrosarcoma, a similar neoplasia both in terms of radiology and histology.…”

“…It can affect adjacent areas, such as pelvic viscera and sacroiliac joints, and more distant sites, such as the lungs, liver and bone. 3,4,8 Despite the low tendency for metastasis to occur, approximately 40% to 60% of the patients develop distant metastases over the course of the disease. 9 Currently, specific preoperative planning techniques, such as three-dimensional (3D) printed osteotomy, may be used for a better anatomical study of the region, with greater precision of the surgical procedure.…”

“…1 They affect the midline along the neuroaxis, with preferential involvement of the sacrum (50% of the cases), followed by the spheno-occipital region (25% to 30%), the cervical vertebrae (10%), and the thoracolumbar vertebrae (5%). [2][3][4] Due to its invasive behavior, the lesion may infiltrate adjacent muscles, sacral nerve roots, pelvic viscera, and sacroiliac joints. 5 They correspond to 1% to 4% of primary bone malignancies, representing the most common primary malignant bone tumor of the spine, 2,5 with male predominance in a ratio of 2:1.…”

“…1 They affect the midline along the neuroaxis, with preferential involvement of the sacrum (50% of the cases), followed by the spheno-occipital region (25% to 30%), the cervical vertebrae (10%), and the thoracolumbar vertebrae (5%). 2 3 4 Due to its invasive behavior, the lesion may infiltrate adjacent muscles, sacral nerve roots, pelvic viscera, and sacroiliac joints. 5…”

“…They correspond to 1% to 4% of primary bone malignancies, representing the most common primary malignant bone tumor of the spine, 2 5 with male predominance in a ratio of 2:1. 1 The peak incidence is between 50 and 60 years of age, 2 4 5 and children and adolescents are rarely affected (< 5% of the cases). 2 Chordoma has a relatively high mortality rate, with an average life expectancy of 5 to 7 years after the diagnosis, abbreviated to 1 year in individuals not submitted to adequate treatment.…”